Evidence map›Paper›PMID 40145682›Full record

ArticleJournal of clinical apheresis2025

A Descriptive 5-Year Analysis of the Demographics and Therapies for Patients With Immune Thrombotic Thrombocytopenic Purpura in the USA: A Multicenter Study of 390 Disease Episodes From 2017 to 2021.

Jeremy W Jacobs, Brian D Adkins, Garrett S Booth, Caroline G Stanek, Elizabeth S Allen, Brenda J Grossman, Laura D Stephens, Elizabeth P Crowe, Laetitia Daou, Marisa B Marques and 25 more

Abstract readMulticenter Study
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of clinical apheresis, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

35 authors.

Jeremy W JacobsDepartment of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.ORCID https://orcid.org/0000-0002-5719-9685
Brian D AdkinsDivision of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.ORCID https://orcid.org/0009-0005-7961-0083
Garrett S BoothDepartment of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.ORCID https://orcid.org/0000-0002-8422-9480
Caroline G StanekDepartment of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA.ORCID https://orcid.org/0009-0006-4274-2354
Elizabeth S AllenDepartment of Pathology, University of California San Diego, La Jolla, California, USA.
Brenda J GrossmanDepartment of Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA.ORCID https://orcid.org/0000-0002-1500-8211
Laura D StephensDepartment of Pathology, University of California San Diego, La Jolla, California, USA.ORCID https://orcid.org/0000-0002-9101-9326
Elizabeth P CroweDivision of Transfusion Medicine, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.ORCID https://orcid.org/0000-0003-1910-2800
Laetitia DaouDivision of Transfusion Medicine, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Marisa B MarquesDepartment of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA.ORCID https://orcid.org/0000-0002-6689-7856
Rance C SiniardDepartment of Pathology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Lisa R WallaceDepartment of Pathology & Immunology, Washington University School of Medicine, St. Louis, Missouri, USA.
Chisa YamadaDepartment of Pathology, Division of Transfusion Medicine, University of Michigan, Ann Arbor, Michigan, USA.ORCID https://orcid.org/0000-0002-1968-2385
Miriam Andrea DuqueDepartment of Pathology and Laboratory Medicine, University of Miami Miller School of Medicine, Miami, Florida, USA.
Yanyun WuDepartment of Pathology and Laboratory Medicine, University of Miami Miller School of Medicine, Miami, Florida, USA.
Omar AljubooriDepartment of Pathology and Laboratory Medicine, University of Miami Miller School of Medicine, Miami, Florida, USA.
Thomas J HarringtonDivision of Hematology, Department of Medicine, University of Miami Miller School of Medicine, Miami, Florida, USA.
Diana M ByrnesDivision of Hematology, Department of Medicine, University of Miami Miller School of Medicine, Miami, Florida, USA.
Quentin EichbaumDepartment of Pathology, Microbiology & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
Cristina A Figueroa VillalbaDepartment of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA.
Justin E JuskewitchDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.ORCID https://orcid.org/0000-0002-7868-2852
Ellen KlapperDivision of Transfusion Medicine, Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California, USA.
Ingrid Perez-AlvarezDivision of Transfusion Medicine, Department of Pathology and Laboratory Medicine, Cedars-Sinai Medical Center, Los Angeles, California, USA.ORCID https://orcid.org/0000-0001-8600-7454
Monica S KleinDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Fatima AldarweeshDepartment of Pathology, University of Chicago, Chicago, Illinois, USA.
Rahaf AlkhatebDepartment of Pathology, University of Chicago, Chicago, Illinois, USA.
Meredith G ParsonsDepartment of Pathology, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.ORCID https://orcid.org/0000-0001-9979-1938
Annette J SchlueterDepartment of Pathology, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.ORCID https://orcid.org/0000-0003-2442-7079
Christopher A TormeyDepartment of Laboratory Medicine, Yale School of Medicine, New Haven, Connecticut, USA.ORCID https://orcid.org/0000-0003-1785-2245
Allison P WheelerWashington Center for Bleeding Disorders, Seattle, Washington, USA.
Amy A PowersDepartment of Pathology, The Queen's Medical Center, Honolulu, Hawaii, USA.
Christopher B WebbDivision of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.
Sean G YatesDivision of Transfusion Medicine and Hemostasis, Department of Pathology, University of Texas Southwestern Medical Center, Dallas, Texas, USA.ORCID https://orcid.org/0000-0001-9703-5047
Evan M BlochDivision of Transfusion Medicine, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.ORCID https://orcid.org/0000-0001-8181-9517
Jay S RavalDepartment of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.ORCID https://orcid.org/0000-0001-9835-957X

Funding

The Malaria Transfusion Risk (MATRix) StudyK23HL151826 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI BLOCH, EVAN MARTIN · 2020 to 2024
$882k
National Heart Lung and Blood Institute 1K23HL15182NHLBI NIH HHS K23 HL151826
6 · The paper itself

Abstract

Immune thrombotic thrombocytopenic purpura (iTTP) is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular occlusion secondary to acquired ADAMTS13 deficiency. Contemporary data regarding iTTP treatment practices in the US, including the use of caplacizumab, are lacking. We aimed to characterize the demographics and therapies, including medications and apheresis practices, in patients with iTTP in the US. We retrospectively analyzed iTTP cases at 15 sites in the US that provide comprehensive care for patients with iTTP. The time-period assessed was from January 1, 2017 to December 31, 2021. Our primary objective was to analyze data by iTTP episode, inclusive of initial episodes and relapses. A total of 390 iTTP episodes were reported for 280 unique individuals (187 females, 93 males). Thirty-day mortality was 3.7% (14/374), and 6-month mortality was 7.4% (27/367). TPE details were reported for 343 episodes, among which 261 underwent at least one procedure (median 6, IQR 3-11). Among the 261 episodes with at least one therapeutic plasma exchange (TPE) performed, 82.0% (214/261) used only plasma. Caplacizumab was used either alone or in combination with other agents in 43 (11.0%) episodes. Management strategies for iTTP remain varied across centers in the US, with a variety of combinations for TPE replacement fluids and therapeutic agents, as well as limited use of caplacizumab. Further research and standardization of treatment regimens may further reduce mortality in this condition.

Indexed as

Blood Component RemovalPurpura, Thrombotic ThrombocytopenicADAMTS13 ProteinAdolescentAdultAgedFemaleHumansMaleMiddle AgedPlasma ExchangeRetrospective StudiesSingle-Domain AntibodiesUnited StatesYoung AdultADAMTS13 ProteinADAMTS13 protein, humancaplacizumabSingle-Domain Antibodiesapheresiscaplacizumabepidemiologyhematologytherapeutic plasma exchangethrombotic thrombocytopenic purpuratransfusion medicine

Identifiers

PMID40145682
PMCPMC11948952

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.